Acquired hepatocerebral degeneration

Fermina Pirmohamed MD (Dr. Pirmohamed of Virginia Commonwealth University School of Medicine has no relevant financial relationships to disclose.)Leslie J Cloud MD MSc (Dr. Cloud of Virginia Commonwealth University School of Medicine and Director of the Parkinson's Disease Program at VCU Parkinson's and Movement Disorders Center has no relevant financial relationships to disclose.)Joseph Jankovic MD, editor. (

Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, has received research and/or training grants from Adamas, Allergan, Biotie, Civitas/Acorda, Hoffmann-La Roche, Medtronic, Merz, Neurocrine , Nuvelution, Pfizer, Prothena, Psyadon, Revance, and Teva; and has served as a consultant or as an advisory committee member for Adamas, Allergan, Merz, Prothena, Revance, and Teva.

)Originally released September 16, 2007; last updated February 27, 2017; expires February 27, 2020

This article includes discussion of acquired hepatocerebral degeneration, chronic acquired hepatocerebral degeneration, and non-Wilsonian hepatocerebral degeneration. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

A minority of patients with chronic liver disease develop acquired hepatocerebral degeneration – a progressive neurologic disorder characterized by extrapyramidal signs, ataxia, and cognitive decline. Although the pathogenesis of acquired hepatocerebral degeneration is not known, diversion of portal blood into the systemic circulation appears to underlie the syndrome. In this article, the authors review the clinical, radiological, and pathological features of acquired hepatocerebral degeneration along with current treatment strategies. Evidence supporting and opposing the potential role of manganese in acquired hepatocerebral degeneration s pathogenesis is discussed, as well as outcomes following liver transplantation.

Key points

• Acquired hepatocerebral degeneration is a neurologic disorder that is clinically distinct from hepatic encephalopathy and characterized by extrapyramidal signs, ataxia, and cognitive dysfunction.

• The pathogenesis of acquired hepatocerebral degeneration is not known, but portosystemic shunting likely plays a central role.

• Acquired hepatocerebral degeneration is not a contraindication to liver transplantation; indeed, in some cases transplantation may be the only effective therapy.

Historical note and terminology

In 1912, S A Kinnear Wilson s manuscript on hepatolenticular degeneration aroused new interest in a possible association between liver dysfunction and neurologic disease (Wilson 1912). The progress that followed marks 1 of neurology s greatest successes in translating a clinical observation into molecular understanding and cure of an otherwise fatal neurodegenerative disorder. Wilson disease is now recognized to be a hereditary disorder of biliary copper excretion. Once inescapably fatal, it is now readily treatable—and often reversible (Lorincz 2010). In contrast to Wilson disease, most cirrhotic patients with progressive neurologic dysfunction have non-inherited (acquired) types of hepatic failure that do not respond to copper-lowering drugs. Acquired hepatocerebral degeneration, first described by vanWoerkem in 1914 (vanWoerkem 1914), was largely unrecognized until the landmark review by Victor, Adams, and Cole in 1965 (Victor et al 1965).

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